PBL #14 – Astro Boy
What do we know?
Timothy J – male - 4.5 months old
Two week history of gradually increasing shortness of breath (SOB)
Distressed on minimal exertion such as suckling
10 year old sister and 8 year old brother
Father, William, a lawyer… Mother, Bernice, part time accountant
Normal birth, 3.5kg, Apgar score of 10
Has had a persistent nasal discharge since birth
Failed to gain weight despite good appetite of breast milk
Respiratory tract infection at 10 weeks – responded to antibiotics
Had Otitis Media shortly after the RTI – responded to antibiotics
Recurred Otitis Media 2 weeks later – responded to antibiotics
Developed diarrhea over the past 6 weeks – soiling his nappies 8-20x per day
5 Hospital admissions since birth, the last one was for IV fluid replacement
Results of Examination
Respiratory rate 40 /min
BP 85/55
Heart rate 160/min
Temp 37.8 o C
Weight 3.6 Kg
Weight is less than the first percentile for his age
Muscle wasting
Irritable and cyanosed
White plaques from tongue
Bucca mucos and Muco-pirulent discharge from nose
Intercostal recession
Tracheal Tug
Audible wheeze and widespread crackles over lung fields
Tempanic membrane bilaterally inflamed
Yellow liquid faecies
Excoriated genital rash extending to anus
CXR - bilateral diffuse opacities throughout both lung fields in an interstitial pattern
Radiologist commented that CXR shows an infection pattern of Pneumocytis Carinii
No thymic shadow on CXR
Nasopharyngeal Aspirate – gram negative bacilli on gram stain
Culture grew to Pseudonomas Aeruginosa
No parasites in faecies
Latex agglutination for Rotavirus - positive
Oral and groin scraping showed Candida Albicans
Neutrophil Respiratory Burst test 95% (normal > 90%)
DNA testing for cystic fibrosis - negative
Electron Microscopy of Cilia – normal
HIV Antibody – negative
Serum biochemistry and blood gases were consistent with severe dehydration and
respiratory failure requiring mechanical ventilation
Timothy's haematology results were:
Hb . . . . . 80.0 (100-140 g/l)
WCC . . . 4.8 (4.0-11.0) x109
Lymphocytes . 0.76 (2.2-7.0*) x 109
*This reference range may vary from one laboratory to another, but note that it is
higher than in adults.
Immunoglobulins:
IgG . . 1.7 (1.7-8.1) g/l
IgA . . <0.01 (0.04-0.84) g/l
IgM . .< 0.01 (0.27-1.0) g/l
(Age adjusted reference ranges allow for lowest to highest concentrations from 4-5
months of age)
Lymphocyte subsets:
CD3 . . . <0.01 (1.7-3.6) x109 . . (all T cells)
CD4 . . . <0.01 (1.7-2.8) x109. . (helper-inducer T cells)
CD8 . . . <0.01 (0.8-1.2) x109. . (cytotoxic/suppressor T cells)
CD19 . . 0.75
(0.5-1.5) x109. . (all B cells)
CD16 . . 0.07
(0.3-0.7) x109. . (Natural Killer cells)
Complement concentration and activity
C3 . . . 1.6 (0.9-1.8) g/l
C4 . . . 0.31 (0.16-0.50) g/l
CH50 . . 578 (520-660) U/ml RI is method dependant.
What do we want to know?
Full medical history
Family history
Birth history
Any known allergies
Any medications
Why has the mother only brought him in today?
Number of nappies
Associated symptoms like fever
Growth chart
Behaviour – crying, sleeping well, irritable, lethargic ect.
Problem feeding?
Family life: abuse?
Physical examination
Vital signs
Feeding – suckling – how? what?
Hydration level (dehydrated?)
Antibiotics – type and dose
Full blood count
Blood culture
Chest X Ray
Stool specimen
Urine specimen
Mouth swab / sputum
Liver function
Renal function
Why is he not mounting a febrile response?
Hypothesis
Blocked nose
Cleft palate
Oral Candidiasis – white plaque on tongue
Potential multiple infections or systemic infection
Upper respiratory tract infection
Immuno-suppressed
What info do we need to solve the problem?
Acquired and congenital immune deficiencies
How does the body mount a febrile response